Dandy Walker Syndrome

This syndrome comprises agenesis of the cerebellar vermis with cystic dilatation of the fourth ventricle, enlargement of the posterior fossa and hydrocephalus. The hydrocephalus is often absent at the time of birth but is present in 75% of cases by the age of 3 months. Additional brain malformations are present in over half of the cases. Neurodevelopmental delay is reported in up to 70% of cases.

Some controversy exists among neurosurgeons as to the best surgical treatment of this condition. The principal issue is regarding the placement of the proximal catheter: whether this should be placed in the lateral ventricle, the posterior fossa cyst or indeed whether both compartments should be shunted simultaneously. A further option is to shunt the infraten-torial compartment in the first instance and then to treat the supratentorial hydrocephalus endoscopically if the ventricles fail to decompress.

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